PsART-ID inception cohort: clinical characteristics, treatment choices and outcomes of patients with psoriatic arthritis.

OBJECTIVES: Our aim is to understand clinical characteristics, real-life treatment strategies, outcomes of early PsA patients and determine the differences between the inception and established PsA cohorts. METHODS: PsArt-ID (Psoriatic Arthritis- International Database) is a multicentre registry. From that registry, patients with a diagnosis of PsA up to 6 months were classified as the inception cohort (n==388). Two periods were identified for the established cohort: Patients with PsA diagnosis within 5-10 years (n = 328), ≥10 years (n = 326). Demographic, clinical characteristics, treatment strategies, outcomes were determined for the inception cohort and compared with the established cohorts. RESULTS: The mean (s.d.) age of the inception cohort was 44.7 (13.3) and 167/388 (43.0%) of the patients were male. Polyarticular and mono-oligoarticular presentations were comparable in the inception and established cohorts. Axial involvement rate was higher in the cohort of patients with PsA ≥10 years compared with the inception cohort (34.8% vs 27.7%). As well as dactylitis and nail involvement (P = 0.004, P = 0.001 respectively). Both enthesitis, deformity rates were lower in the inception cohort. Overall, 13% of patients in the inception group had a deformity. MTX was the most commonly prescribed treatment for all cohorts with 10.7% of the early PsA patients were given anti-TNF agents after 16 months. CONCLUSION: The real-life experience in PsA patients showed no significant differences in the disease pattern rates except for the axial involvement. The dactylitis, nail involvement rates had increased significantly after 10 years from the diagnosis and the enthesitis, deformity had an increasing trend over time.

Disease characteristics of psoriatic arthritis patients may differ according to age at psoriasis onset: cross-sectional data from the Psoriatic Arthritis-International Database.

OBJECTIVES: To explore the impact of early versus late-onset psoriasis (PsO) on the disease characteristics of psoriatic arthritis (PsA) in a large-multicentre cohort. METHODS: The data from a multicentre psoriatic arthritis database was analysed. Patients were grouped according to age at psoriasis onset (early onset; <40 years of age, late-onset; >40 years of age) and disease characteristics of the groups were compared by adjusting for BMI and PsA duration, where necessary. RESULTS: At the time of analyses, 1634 patients were recruited [62.8% females; early onset 1108 (67.8%); late-onset, 526 (32.2%)]. The late-onset group was more over-weight [66.8% vs. 86.8%, p<0.001; adjusted for age - aOR 1.55 (1.11-2.20; 95% CI)]. The early onset group had more scalp psoriasis at onset (56.7% vs. 43.0%, p<0.001), whereas extremity lesions were more common in the late-onset group (63.8% vs. 74.2%, p<0.001). Axial disease in males and psoriatic disease family history in females were significantly higher in the early onset group [38.0% vs. 25.4%; p=0.005; adjusted for PsA duration - aOR 1.76 (1.19-2.62; 95% CI) / 39.5% vs. 30.1%; p=0.003; OR 1.51 (1.15-1.99; 95% CI), respectively]. Psoriatic disease activity parameters, patient-physician reported outcomes and HAQ-DI scores were similar in both groups. CONCLUSIONS: Clinical features of PsA may be affected by the age at onset of PsO. Different genetic backgrounds in early and late-onset PsO may be driving the differences in psoriasis and PsA phenotypes.

Criteria sets for primary Sjogren's syndrome are not adequate for those presenting with extraglandular organ involvements as their dominant clinical features.

Patients with primary Sjogren's syndrome (pSS) may go undiagnosed or be misclassified due to the insidious nature and wide spectrum of the disease. The available several classification criteria emphasize glandular findings. We aimed to analyze the efficiency of various classification criteria sets in patients diagnosed on the clinical basis by expert opinion and to compare those pSS patients who fulfilled these criteria with those who did not. This is a multicenter study in which 834 patients from 22 university-based rheumatology clinics are included. Diagnosis of pSS was made on the clinical basis by the expert opinion. In this study, we only interviewed patients once and collected available data from the medical records. The European criteria, American-European Consensus Group (AECG) and American College of Rheumatology (ACR) Sjogren's criteria were applied. Majority of the patients were women (F/M was 20/1). The median duration from the first pSS-related symptom to diagnosis was significantly shorter in men (2.5 ± 2.3 vs 4.3 ± 5.9 years) (p = 0 < 0.016). When the European, AECG and ACR Sjogren's criteria were applied, 666 patients (79.9%) satisfied at least one of them. In total, 539 patients (64.4%) satisfied the European, 439 (52.6%) satisfied the AECG, and 359 (43%) satisfied the ACR criteria. Among the entire group, 250 patients (29.9%) satisfied all and 168 (20.1%) met none of the criteria. The rates of extraglandular organ involvements were not different between patients who met at least one of the criteria sets and those who met none. There is an urgent need for the modification of the pSS criteria sets to prevent exclusion of patients with extraglandular involvements as the dominant clinical features.

Response rate of initial conventional treatments, disease course, and related factors of patients with adult-onset Still's disease: Data from a large multicenter cohort.

BACKGROUND: Adult-onset Still's disease (AOSD) is a rare condition, and treatment choices are frequently dependent on expert opinions. The objectives of the present study were to assess treatment modalities, disease course, and the factors influencing the outcome of patients with AOSD. METHODS: A multicenter study was used to reach sufficient patient numbers. The diagnosis of AOSD was based on the Yamaguchi criteria. The data collected included patient age, gender, age at the time of diagnosis, delay time for the diagnosis, typical AOSD rash, arthralgia, arthritis, myalgia, sore throat, lymphadenopathy, hepatomegaly, splenomegaly, pleuritis, pericarditis, and other rare findings. The laboratory findings of the patients were also recorded. The drugs initiated after the establishment of a diagnosis and the induction of remission with the first treatment was recorded. Disease patterns and related factors were also investigated. A multivariate analysis was performed to assess the factors related to remission. RESULTS: The initial data of 356 patients (210 females; 59%) from 19 centers were evaluated. The median age at onset was 32 (16-88) years, and the median follow-up time was 22 months (0-180). Fever (95.8%), arthralgia (94.9%), typical AOSD rash (66.9%), arthritis (64.6%), sore throat (63.5%), and myalgia (52.8%) were the most frequent clinical features. It was found that 254 of the 306 patients (83.0%) displayed remission with the initial treatment, including corticosteroids plus methotrexate with or without other disease-modifying antirheumatic drugs. The multivariate analysis revealed that the male sex, delayed diagnosis of more than 6 months, failure to achieve remission with initial treatment, and arthritis involving wrist/elbow joints were related to the chronic disease course. CONCLUSION: Induction of remission with initial treatment was achieved in the majority of AOSD patients. Failure to achieve remission with initial treatment as well as a delayed diagnosis implicated a chronic disease course in AOSD.

Evaluation of nailfold videocapillaroscopy in central serous chorioretinopathy.

BACKGROUND: Nailfold videocapillaroscopy (NVC) is a diagnostic tool to evaluate micro-vasculature. The presence of choroidal vasculopathy is apparent in central serous chorioretinopathy (CSCR). OBJECTIVES: This study was aimed at assessing capillaroscopic nailfold findings in patients with CSCR. To the best of our knowledge, there is no study assessing NVC findings in CSCR in the literature. METHOD: Sixty-one patients with CSCR who met the inclusion criteria, and 82 age- and sex-matched healthy controls were included to the study. A videocapillaroscopy device with 200× magnification was used for capillaroscopic assessment. RESULTS: The mean age was 48.79 ± 11.15 years in the patient group (13 female, 48 male) and 49.38 ± 9.02 years in the control group (17 female, 65 male). The age and gender were comparable in the patient and control groups (p = 0.727 and p = 0.933, respectively). The capillary count was found to be decreased in the patient group compared to control group. No significant correlation was found between capillary count and choroidal thickness (p = 0.551; r = -0.081). In the patient group, the frequencies of major capillaroscopic findings including capillary ectasia, aneurysm, micro-hemorrhage, avascular area, tortuosity, neo-formation, bizarre capillary, bushy capillary, meander capillary and extravasation were found to be increased in the patient group. However, no significant correlation was detected between capillaroscopic findings and disease type and presence of attacks. CONCLUSIONS: This is first study in which nailfold capillary assessment was performed in patients with CSCR, and we detected major capillaroscopic changes. These findings suggest that CSCR can be a systemic microvasculopathy. Further studies are needed to clarify the diagnostic and prognostic value of capillaroscopy in CSCR.

A multicenter report of biologic agents for the treatment of secondary amyloidosis in Turkish rheumatoid arthritis and ankylosing spondylitis patients.

In this multicenter, retrospective study, we evaluated the efficacy and safety of biologic therapies, including anti-TNFs, in secondary (AA) amyloidosis patients with ankylosing spondylitis (AS) and rheumatoid arthritis (RA). In addition, the frequency of secondary amyloidosis in RA and AS patients in a single center was estimated. Fifty-one AS (39M, 12F, mean age: 46.7) and 30 RA patients (11M, 19F, mean age: 51.7) with AA amyloidosis from 16 different centers in Turkey were included. Clinical and demographical features of patients were obtained from medical charts. A composite response index (CRI) to biologic therapy-based on creatinine level, proteinuria and disease activity-was used to evaluate the efficacy of treatment. The mean annual incidence of AA amyloidosis in RA and AS patients was 0.23 and 0.42/1000 patients/year, respectively. The point prevalence in RA and AS groups was 4.59 and 7.58/1000, respectively. In RA group with AA amyloidosis, effective response was obtained in 52.2 % of patients according to CRI. RA patients with RF positivity and more initial disease activity tended to have higher response rates to therapy (p values, 0.069 and 0.056). After biologic therapy (median 17 months), two RA patients died and two developed tuberculosis. In AS group, 45.7 % of patients fulfilled the criteria of good response according to CRI. AS patients with higher CRP levels at the time of AA diagnosis and at the beginning of anti-TNF therapy had higher response rates (p values, 0.011 and 0.017). During follow-up after anti-TNF therapy (median 38 months), one patient died and tuberculosis developed in two patients. Biologic therapy seems to be effective in at least half of RA and AS patients with AA amyloidosis. Tuberculosis was the most important safety concern.

The role of nailfold capillaroscopy in interstitial lung diseases - can it differentiate idiopathic cases from collagen tissue disease associated interstitial lung diseases?

INTRODUCTION: Nailfold capillaroscopy (NFC) is a non-invasive diagnostic test that is mostly used for early diagnosis of collagen tissue diseases (CTDs). We aimed to evaluate whether NFC findings could be a clue for discriminating idiopathic interstitial lung diseases (ILD) from CTD associated ILDs (CTD-ILD). Additionally it was aimed to determine whether NFC could be helpful in discriminating usual interstitial pneumonia (UIP) pattern from non-specific interstitial pneumonia (NSIP) pattern. MATERIALS AND METHODS: We grouped patients into three main groups: 15 CTD-ILD, 18 idiopathic ILD, and 17 patients in the control group. The CTD-ILD group was split into two subgroups: 8 patients with Sjögren's syndrome (SJS)-associated ILD and 7 with rheumatoid arthritis (RA)-associated ILD. The idiopathic-ILD group consisted of 10 idiopathic NSIP and 8 IPF patients. The control group consisted of 10 SJS and 7 RA patients without lung disease. None of the patients were on acute exacerbation at the time of examination, and none had Reynaud's phenomenon. RESULTS: Mean capillary density was significantly reduced only in the CTD-ILD group as compared to the control group (p= 0.006). In subgroup analysis, it was determined that RA-ILD, IPF, and SJS-ILD subgroups had more severe capillaroscopic abnormalities. Mean capillary density in patients with the UIP pattern was reduced compared to patients with the NSIP pattern and those in the control group; p values were 0.008 and < 0.001, respectively. CONCLUSION: This study is to be the first describing and comparing the nailfold capillaroscopic findings of patients with NSIP and UIP patterns. NFC findings can be helpful in discriminating UIP patterns from NSIP patterns. But to show its role in differentiating idiopathic disease, more studies with more patients are needed.

Synaptosomal-associated protein 25 (Snap-25) gene polymorphism frequency in fibromyalgia syndrome and relationship with clinical symptoms.

BACKGROUND: SNAP-25 protein is contributory to plasma membrane and synaptic vesicle fusions that are critical points in neurotransmission. SNAP-25 gene is associated with behavioral symptoms, personality and psychological disorders. In addition, SNAP-25 protein can be related to different neurotransmitter functions due to its association with vesicle membrane transition and fusion. This is important because neurologic, cognitive, and psychologic disorders in fibromyalgia syndrome (FMS) can be related to this function. This relationship may be enlightening for etiopathogenesis of FMS and treatment approaches. We aimed to study a SNAP-25 gene polymorphism, which is related to many psychiatric diseases, and FMS association in this prospective study. METHODS: We included 71 patients who were diagnosed according to new criteria and 57 matched healthy women in this study. Both groups were evaluated regarding age, height, weight, BMI, education level, marital and occupational status. A new diagnosis of FMS was made from criteria scoring, SF-36, Beck depression scale, and VAS that were applied to the patient group. SNAP-25 gene polymorphism and disease activity score correlations were compared. RESULTS: Mean age was 38±5,196 and 38.12±4.939 in patient and control groups, respectively (p=0.542). No significant difference was found between groups regarding age, height, weight, BMI, education level, marital or occupational status (p > 0.05). Ddel T/C genotype was significantly higher in the patient group (p = 0.009). MnlI gene polymorphism did not show a correlation with any score whereas a significant correlation was found between Ddel T/C genotype and Beck depression scale and VAS score (p < 0.05). CONCLUSION: FMS etiopathogenesis is not clearly known. Numerous neurologic, cognitive and psychological disorders were found during studies looking at cause. Our study showed increased SNAP-25 Ddel T/C genotype in FMS patients compared to the control group, which is related to behavioral symptoms, personality and psychological disorders in FMS patients.

Demographic and clinical features of gout patients in Turkey: a multicenter study.

Gout results from multifactor interactions between gender, age, genetic and environmental factors. Environmental factors underlying gout and precipitating factors triggering acute attacks might vary in different populations with different lifestyles. In this study, we aimed to collect data regarding the demographic and clinical features, comorbid factors, and precipitating factors associated with the initiation of acute attacks in gout patients in Turkey. A total of 312 patients were included in this study (mean age, 58.8 ± 13.8 years; female/male ratio, 55/257). The demographic features, alcohol intake, clinical and laboratory features, and comorbid conditions including obesity, diabetes mellitus, hyperlipidemia, hypertension, and coronary heart disease were noted in a standard questionnaire. Precipitating factors initiating acute attacks (if any) were also noted. The patients were divided into 4 groups according to the region of location as central Anatolian region, southeast Anatolian region, Aegean region, and Trakya region. Our results were compared according to the gender and the location of the patients. The mean age at the start of the symptoms was 10 years higher in women (60.4 ± 14.8 and 50.6 ± 13.5 years in women and men, respectively, p < 0.001).Obesity was present in 40.1 %, diabetes mellitus in 17.9 %, hyperlipidemia in 30.1 %, hypertension in 53.5 %, coronary artery disease in 17 %, and nephrolithiasis in 21.8 % of patients. Precipitating factors triggering gout flares were as follows: diet (high consumption of meat or fish) in 46.5 %, alcohol consumption in 15.7 %, diuretics in 8.3 %, diet or diuretics in 5.1 %, diet or alcohol in 4.5 %, diet or alcohol or diuretics in 1.6 %, others in 4.2 %, and none in 14.1 %. The presence of diabetes and diuretic use was more common among women. Use of diuretics is a more common trigger for gout flares among women. On the other hand, various comorbid conditions, such as obesity and hypertension, and triggers for gout flares may differ between patients living in different geographic regions. In summary, we reported the first data regarding clinical and demographic characteristics of gout in Turkey. The majority of our patients could describe at least one "trigger" that initiated gout flare. Both comorbid conditions and triggers of attack might differ between men and women, and in different geographic areas. Better knowledge of the modifiable risk factors can be useful for the management strategy to optimize long-term patient outcomes in local clinics.

Budd-Chiari syndrome in Behçet's disease: a retrospective multicenter study.

OBJECTIVE: To compare the clinical features, laboratory findings, and prognosis of Behçet's disease (BD) patients with and without Budd-Chiari syndrome (BCS). METHODS: This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017. RESULTS: Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14-47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%). CONCLUSION: To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points • Mortality rate is higher in BD-associated BCS patients with IVC involvement. • Chronic and silent form of BD-associated BCS has a better prognosis. • The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial.

Assessment of Microcirculation in HIV-Positive Patients with a Noninvasive Method: Nailfold Videocapillaroscopy.

Chronic inflammation that contributes to the deterioration of the mechanical form of both the vascular wall and the function of endothelial has been shown to cause vascular dysfunction in patients with human immunodeficiency virus (HIV). Nailfold videocapillaroscopy (NVC) is a noninvasive and simple technique used to analyze dermal microvasculature. The aims of our study were to detect the morphological and functional properties of microcirculation in HIV-positive patients utilizing NVC and to compare the results with those obtained from healthy individuals and investigate whether these changes had supported any correlation with HIV. In this prospective study, 51 male HIV-positive patients were the subjects and 59 male healthy individuals made up the control group. Capillaroscopic findings, obtained from both groups by NVC, were evaluated. Tortuosity, a capillaroscopic finding, was significantly higher in the group with HIV (p < .001). In logistic regression analysis, tortuosity [odds ratio (OR), 4.658; p = .001], bizarre capillary (OR, 1.733; p = .319), and capillary ectasia (OR, 0.867; p = .493) were considered significant. The changes of capillaroscopic findings were found to be correlated with HIV. We suggest videocapillaroscopy in the evaluation of microvascular changes in HIV patients, as a dependable contributing method.

Triple or quadruple tetracycline-based therapies versus standard triple treatment for Helicobacter pylori treatment.

BACKGROUND: Our aim was to compare lansoprazole-tetracycline-metranidazole (LTM) as first-line treatment with the classical lansoprazole-amoxicillin-clarithromycin (LAC) and bismuth-containing quadruple treatments. PATIENTS AND METHODS: This prospective, single-center, randomized study included 464 consecutive Helicobacter pylori-positive patients with dyspeptic symptoms. A total of 415 patients completed the study. The patients were allocated into 4 study groups using random sampling numbers as follows-LAC group: lansoprazole 30 mg twice daily, amoxicillin 1000 mg twice daily, and clarithromycin 500 mg twice daily for 14 days; BLTM group: bismuth subcitrate 300 mg 4 times a day, lansoprazole 30 mg twice daily, tetracycline 500 mg 4 times a day, and metronidazole 500 mg twice daily for 10 days; RBLTM group: ranitidine bismuth citrate 400 mg twice daily, lansoprazole 30 mg twice daily, tetracycline 500 mg 4 times a day, and metronidazole 500 mg twice daily for 10 days; and LTM group: lansoprazole 30 mg twice daily, tetracycline 500 mg 4 times a day, and metronidazole 500 mg twice daily for 10 days. RESULTS: The per protocol H. pylori eradication rate in LAC, BLTM, RBLTM, and LTM groups were 37 of 104 (35.6%), 56 of 102 (54.9%), 67 of 104 (64.4%), and 63 of 105 (60%), respectively. The intention-to-treat eradication rate was 37 of 113 (32.7%) in LAC, 56 of 119 (47.1%) in BLTM, 67 of 117 (57.3%) in RBLTM, and 63 of 115 (54.8%) in LTM group. The BLTM, RBLTM, and LTM treatment groups achieved a significantly better eradication rate than the LAC treatment group (P < 0.001). There was not any significant statistical difference between the groups of BLTM, RBLTM, and LTM. CONCLUSION: LTM treatment group achieved a significantly better eradication rate than the LAC treatment group. The success ratio of LTM therapy is comparable with quadruple bismuth-based treatments.

Bone mineral density and serum osteocalcin levels in patients with gout.

Objective: The objective of the study is to evaluate the relation of gout with osteoporosis and serum osteocalcin (OC) levels. Material and methods: Seventy-five patients diagnosed with gout and 55 controls were included in the study. Comorbid conditions and drugs associated with osteoporosis were excluded. The T and Z scores from lumbar spine (L2-L4) and femur (neck, ward, trochanter, total) were determined by dual-energy X-ray absorptiometry (DXA). OC levels were measured by enzyme-linked immunosorbent assay. Results: Osteoporosis according to T scores of lumbar vertebrae L2-L4 was found to be significantly higher in patients with gout compared to the control group (p = 0.02). Lumbar spine T-score was -1.6 in gout group and -1.0 in controls. OC level was 7.9 ng/mL in the gout group and 18.9 ng/mL in the control group. There was a significant difference (p < 0.001). In addition, mean OC level was 12.4 ± 6.9 ng/mL in the patients diagnosed with osteoporosis and 17.2 ± 10.6 ng/mL in the patients that were classified as normal and a significant difference was established between the two groups (p = 0.03). A significant negative correlation was found between OC level and body mass index, age, and age at first attack. Similarly, femoral T-score established a negative correlation with parathyroid hormone, age, age at first attack, and allopurinol dose. Conclusion: Serum OC level can be a useful marker in the assessment of bone turnover and clinicians should keep osteoporosis in mind in gout patients.

Frequency of Erectile Dysfunction in Males with Central Serous Chorioretinopathy.

OBJECTIVES: To evaluate the frequency of sexual dysfunction and associated factors in patients with central serous chorioretinopathy (CSCR). MATERIAL AND METHODS: Fifty-eight CSCR patients who met the inclusion criteria and 99 age- and sex-matched healthy controls were prospectively investigated for sexual dysfunction and associated factors. All participants were investigated using the Beck Depression Inventory (BDI), Beck Anxiety Scale (BAS), and the15-question Index of Erectile Function-15 (IIEF-15) and by extensive examination of CSCR, associated factors, and confounding factors. RESULTS: The mean ages of the patient and control groups were 46.95±11.27 and 45.3±10.93 years, respectively (p=0.370). The erectile function, orgasmic function, sexual desire, sexual satisfaction, and overall sexual satisfaction scores of the patient group were significantly lower than those of the control group (p<0.001). Severe erectile dysfunction (ED) was diagnosed in 3 (5.2%), moderate ED in 10 (17.2%), mild-to-moderate ED in 7 (12.1%), and mild ED in 15 (25.9%) patients in the patient group, whereas no severe or moderate ED was diagnosed in the control group. The erectile function score was found to be negatively correlated with age and BMI. No sexual parameters were found to be correlated with choroidal thickness (CT) in either the patient or control group. CONCLUSION: CSCR patients experience a higher incidence of sexual dysfunction compared to healthy individuals without CSCR. The exclusion of confounding factors suggests that factors involved in CSCR pathogenesis may also play a role in ED.

A variant allele of the Mediterranean-fever gene increases the severity of gout.

BACKGROUND: Gout is a clinical syndrome that occurs as an inflammatory response to increased concentration of uric acid and monosodium urate crystals. Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease with autosomal recessive inheritance. The Mediterranean fever (MEFV) gene is responsible for FMF and encodes pyrin that suppresses the inflammatory response. Most of the FMF-related mutations have been identified in exon 2 (e.g., E148Q and R202Q) and exon 10 (M680I, M694V, M694I and V726A) of the MEFV gene, and each missense mutation is known to increase production of interleukin-1, a proinflammatory cytokine. Our aim was to investigate effects of MEFV variant alleles on the manifestations of gout. METHODS: Seventy-one patients diagnosed with gout (age: 61.73 ± 11.73 years, F/M: 14/57) and 50 healthy subjects (age: 61.48 ± 11.97, F/M: 10/40) as controls were included in this study. RESULTS: MEFV variant alleles were found in 24 (33.8%) of the gout patients and in 13 (26%) of the control subjects; the difference was not statistically significant. In the gout patients with a MEFV variant allele, the interval between the first two attacks was shorter (P = 0.014), and the platelet count was higher (P = 0.026), compared to the patients without a variant allele. In addition, the patients with a MEFV variant allele showed the higher incidence of tophus (8.5% vs. 1.4%) (P = 0.005) and the higher number of attacks per year (P = 0.001). CONCLUSION: We propose that a variant allele of the MEFV gene may be responsible for the severity of gout.

Incidence of sleep disturbances in patients with familial Mediterranean fever and the relation of sleep quality with disease activity.

AIM: To evaluate the sleep quality and the relation of sleep quality with depression, anxiety, fatigue and disease activity in adult patients with familial Mediterranean fever (FMF). METHOD: One hundred and seventy-four FMF patients and 84 age-sex matched healthy individuals were included in this study. The Pittsburgh Sleep Quality Index (PSQI), Multidimensional Assessment of Fatigue (MAF) and the Hospital Anxiety and Depression Index (HADS) were used to assess sleep quality, fatigue, depression and anxiety, respectively. RESULT: FMF patients had significantly higher depression, anxiety, fatigue and PSQI scores than healthy controls. As the severity of the disease increased, scores of total PSQI and its domains increased. Patients with total PSQI score higher than 5 had statistically significantly higher erythrocyte sedimentation rates (ESR), serum C-reactive protein and serum amyloid levels during attacks, more attack numbers in last 3 months and worse fatigue, depression scores. Total PSQI score was positively correlated with inflammatory markers during attacks, attack numbers in the last 3 months and fatigue score. Logistic regression models identified disease duration, ESR during attacks, fatigue, attack numbers in the last 3 months as predictors of poor sleep quality. CONCLUSION: Poor sleep quality is common in adult FMF patients. Anxiety, depression and fatigue are more frequent in FMF patients than healthy individuals. Poor sleep quality is associated with inflammatory marker levels during attacks, fatigue and attack numbers in the last 3 months.

Axial psoriatic arthritis: the impact of underdiagnosed disease on outcomes in real life.

Psoriatic arthritis (PsA) may affect different joints, including the spine. The prevalence of spinal involvement is variable depending on the definition and a subset of patients have been identified in cohorts that do not have clinical features of axial disease and yet have imaging findings. Still, there is not a consensus on how and when to screen axial disease. In this study, we aimed to investigate factors associated with being underdiagnosed for axial psoriatic arthritis (axPsA) and its impacts on outcomes. Disease features and outcomes of axPsA according to the physician (n = 415) were compared with patients with imaging findings only (sacroiliitis fulfilling the modified New York criteria, n = 112), using data from a real-life PsA registry. Patients with imaging findings only were more frequently women (83/220 (37.7%) vs 29/122 (23.8%); p = 0.008). This group also had higher peripheral disease activity (imaging only vs clinical AxPsA: mean (SD) tender joint count 5.3 (6.1) vs 3.3 (4.7), swollen joint count 1.9 (2.9) vs 1.2 (2.4); p < 0.001 for both comparisons) and was less often treated using TNF inhibitors (16.1 vs 38.2%; p < 0.001) than patients who were classified as axPsA. Patient-reported outcomes were similar in both groups. PsA patients, especially women with more severe peripheral disease, have a higher risk of being underdiagnosed for axPsA. The severity of peripheral symptoms may be a risk factor to mask the spinal features of PsA.

Nationwide Experience With Off-Label Use of Interleukin-1 Targeting Treatment in Familial Mediterranean Fever Patients.

OBJECTIVE: Approximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients. METHODS: In all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed. RESULTS: In total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced. CONCLUSION: Anti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.

Chronic Non-Productive Cough in Patients With Primary Sjögren's Syndrome.

OBJECTIVES: This study aims to evaluate the frequency of chronic cough due to dryness in the trachea and the relationship between depression and chronic cough in patients with primary Sjögren's syndrome (pSS). PATIENTS AND METHODS: Eighty non-smoking patients (7 males, 73 females; mean age 47.6±9.2 years; range 18 to 70 years) with newly diagnosed pSS were included. All patients were evaluated clinically, radiologically, and physiologically. Patients with cough were assessed using the Leicester Cough Questionnaire and visual analog scale. Beck Depression Inventory was used to determine the risk of depression in patients with cough. Patients with asthma, rhinosinusitis, gastrooesophageal reflux, and drug use which cause cough and pulmonary involvement in pSS were excluded. RESULTS: Non-productive cough was detected in 30 patients (37.5%). Fourteen patients (17.5%) were admitted to the hospital with cough at least once before the diagnosis of pSS. The median time between onset of symptoms and firm diagnosis was 24 months (25-75 interquartile ranges: 3-60 months). A significant difference was observed in the Beck Depression Inventory scores between patients with and without cough. There was a significant negative relationship between Leicester Cough Questionnaire and Beck Depression Inventory scores. CONCLUSION: Patients with pSS often have symptoms for a long duration that mimic those of more commonly encountered non-specific pulmonary conditions. Xerotrachea should be considered in the differential diagnosis of chronic cough, especially in patients with normal pulmonary function tests and pulmonary imaging.

Frequency of fibromyalgia syndrome in patients with central serous chorioretinopathy.

PURPOSE:: To investigate frequency of fibromyalgia syndrome (FMS) among patients with central serous chorioretinopathy (CSCR). METHODS:: The study included 83 patients with CSCR and 201 age- and sex-matched healthy controls; the mean age was 47.5 ± 11.3 years in the CSCR group (18 women; 21.7%) and 47.2 ± 11.2 years in the control group (44 women; 21.9%). All participants were assessed for FMS based on 2010 American College of Rheumatology diagnostic criteria and for depression and anxiety with the Beck Anxiety Inventory (BAI) and Beck Depression Inventory (BDI). RESULTS:: FMS was diagnosed in 35 patients (42.2%) from the CSCR group and in 21 individuals (10.4%) from the control group (p<0.001). It was found in 77.77% of the women (14/18) and 32.3% of the men (21/65) in the CSCR group and in 22.7% of the women (10/44) and 7.0% of the men (11/157) in the control group. Familial stress, BDI and BAI scores were higher in the patients with FMS than in those without. When independent risk factors were evaluated by logistic regression analysis, it was found that only the presence of familial stress was a significant risk factor for FMS. CONCLUSIONS:: Patients with CSCR should be assessed for the presence of FMS, and this should be taken into consideration when developing a treatment plan. Further studies with a larger sample size are needed to clarify the relationship between FMS and CSCR.